Intrapartum management of a patient with urticaria pigmentosa.

Urticaria pigmentosa is a cutaneous variant of mastocytosis. This is a complex group of diseases distinguished by abnormal aggregation of mast cells within the skin and other organs. Mast cell degranulation and subsequent release of vasoactive amines may occur in response to a variety of non-immune triggers leading to, as its most severe manifestation, a clinical picture of anaphylactic shock. We present the anaesthetic management during labour of a patient with urticaria pigmentosa.